Wales Daily Post – 26th November 2002
A special implant has saved a North Wales woman whose heart literally stopped when her toddler screamed.
Nurse Rachel Wiln, 29, was diagnosed in march as having the same abnormal gene that killed her sister, Lisa, just over four years ago.
Countess of Chester Hospital pediatric nurse Lisa suffered from a genetic condition, Long QT Syndrome, which meant her heart could stop if she heard a sudden sound.
It is suspected the sound of Lisa’s alarm clock, which had been set to go off at 6am on the morning of her death, could have proved fatal.
Rachel was eventually persuaded to undergo tests for Long QT Syndrome after her mother, Doreen Harley, sent her articles from the Daily Post highlighting the plight of another North Wales family which had suffered 16 similar deaths.
Former policeman Neil Halliday, 46, from St Asaph, had researched his family and found six of his mother’s brothers and sisters had died under the age of 21. Seven of his cousins had died suddenly and in the third generation three had already died and another five suffered blackouts. see Our family curse
In June, Rachel, who now lives with her family in Nuneaton, had surgery to fit a kind of pacemaker, called an ICD.
The operation was necessary because she reacted badly to drug treatment.
Mrs Harley said:” In August, she was fast asleep in bed when her two-year-old woke up screaming and the sudden noise caused Rachel’s heart to stop and she blacked out. The ICD kick-started her heart and brought her back again.
“If we had not had the tests done we would have lost Rachel.”
DNA tests now confirm Rachel’s eldest son, Jack age 6, definitely has Long QT Syndrome. DNA tests on her younger son, Adam age 2, are due soon.
“We tragically lost Lisa, but at least her death has saved Rachel and her children by having screening.” Mrs Harley said.
Lisa was found dead in bed in January 1998. The pathologist who carried out the post mortem could find no obvious cause of death and recorded an open verdict.
Prior to her death at the age of 27, Lisa had gone to the doctor complaining of palpitations, chest pains and light-headedness. She was dignaosed with stress and depression and was prescribed anti-depressants – drugs on a banned list for anyone suffering from Long QT syndrome.
Research by Mrs Harley led her to St George’s Hospital, London where she and her husband Terry were screened and Terry was found to have the genetic abnormal gene which causes Long QT Syndrome.
His condition is being monitored and he will need beta-blockers if the symptoms begin to show.
The death of her daughter spurred Mrs Harley on to join the charity Cardiac Risk in the Young to raise awareness and campaign for a national screening programme.
The charity has now fixed its first North Wales mobile cardiac screening session which will offer 37 free appointments to young people between 14 and 35. The event will be held at Beaufort Park Hotel in Mold on Saturday, February 22.
The screenings are free to the limited number of young people and the results are confidential.
It will consist of an ECG, an ECHO and a consultation with a specialist cardiologist. If any abnormality is found the client will be invited to St Georges’ Hospital a centre of excellence for cardiac abnormalities, for evaluation.
Mrs Harley said:” We do not want to spread alarm of panic, we just want people to be aware and to recognise the advantages of screening.
“Screening has saved Rachel’s life. It is worth it. I just don’t want any family to suffer the tragedy we have had.”
